中国实用儿科杂志

中国实用儿科杂志 ›› 2022, Vol. 37 ›› Issue (10): 736-739.DOI: 10.19538/j.ek2022100605

• 专题笔谈 • 上一篇    下一篇

糖原累积病的饮食与营养干预

  

  1. 1.河南省儿童医院内分泌遗传代谢科，河南  郑州  450000；2.河南中医药大学第一附属医院儿科， 河南  郑州  450000；3.北京大学第一医院儿科，北京  100034
  • 出版日期:2022-10-06 发布日期:2022-11-11
  • 通讯作者: 卫海燕，电子信箱：haiyanwei2009@163.com
  • 基金资助:
    国家重点研发计划（2019YFC1005100）

Dietary and nutritional intervention in glycogen storage disease

  1. *Department of Endocrinology and Inherited Metabolic，Henan Children’s Hospital，Zhengzhou  450000，China
  • Online:2022-10-06 Published:2022-11-11

摘要： 糖原累积病（glycogen storage disease，GSD）是由于先天性酶缺陷所造成的一组糖原代谢异常，患儿肝脏、肌肉、心肌组织内糖原累积，导致多种疾病，大多缺乏特效治疗，饮食和营养干预可改善部分糖原累积病患者的代谢紊乱，保证患者的生长发育，提高生存质量。应重视糖原累积病的长期营养管理。

关键词: 糖原累积病, 饮食, 营养干预

Abstract: Glycogen storage diseases（GSD）is a group of abnormal glycogen metabolism caused by congenital enzyme defects，and glycogen accumulation in the liver，muscle，myocardial tissue leads to a variety of diseases. Most of them lack specific treatment.Diet and nutritional intervention can improve the metabolic disorders of patients with glycogen storage diseases，ensure the growth and development of patients，and improve the quality of life.Attention should be paid to the long-term nutritional management of glycogen storage diseases.

Key words: glycogen storage disease, diet, nutritional intervent